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. 2017 Aug 3;101(2):239–254. doi: 10.1016/j.ajhg.2017.07.005

Figure 4.

Figure 4

MRPS34 Mutations Are Associated with Reduced Protein Levels of Small Mitoribosomal Subunits and Destabilization of the Mitoribosome

(A and B) SDS-PAGE western blot of protein from fibroblasts showed reduced protein levels of small mitoribosomal subunit proteins in subjects 1 (A) and 4 (B) relative to control individuals (C1 and C2). The abundance of large mitoribosomal proteins in subjects 1 and 4 were comparable to control individuals. Complex II subunit SDHA, VDAC1, and GAPDH were used as loading controls.

(C) A continuous 10%–30% sucrose gradient was used to determine the distribution of the small and large ribosomal subunit and the monosome in mitochondria isolated from control individual (C) and subject 1 cells. Mitochondrial ribosomal protein markers of the small (MRPS16 and MRPS35) and large (MRPL11 and MRPL37) ribosomal subunits were detected by immunoblotting with specific antibodies. The data are representative of results from three independent biological experiments. The dashed vertical lines denote the relevant fractions as indicated.